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Abstract
Objective: We report the clinical and serologic features of Japanese patients with chronic inflammatory demyelinating polyneuropathy (CIDP) displaying anti-neurofascin-155 (NF155) immunoglobulin G4 (IgG4) antibodies.
Methods: In sera from 533 patients with CIDP, anti-NF155 IgG4 antibodies were detected by ELISA. Binding of IgG antibodies to central and peripheral nerves was tested.
Results: Anti-NF155 IgG4 antibodies were identified in 38 patients (7%) with CIDP, but not in disease controls or normal participants. These patients were younger at onset as compared to 100 anti-NF155–negative patients with CIDP. Twenty-eight patients (74%) presented with sensory ataxia, 16 (42%) showed tremor, 5 (13%) presented with cerebellar ataxia associated with nystagmus, 3 (8%) had demyelinating lesions in the CNS, and 20 of 25 (80%) had poor response to IV immunoglobulin. The clinical features of the antibody-positive patients were statistically more frequent as compared to negative patients with CIDP (n = 100). Anti-NF155 IgG antibodies targeted similarly central and peripheral paranodes.
Conclusion: Anti-NF155 IgG4 antibodies were associated with a subgroup of patients with CIDP showing a younger age at onset, ataxia, tremor, CNS demyelination, and a poor response to IV immunoglobulin. The autoantibodies may serve as a biomarker to improve patients' diagnosis and guide treatments.
GLOSSARY
- Caspr1=
- contactin-associated protein-1;
- CCPD=
- combined central and peripheral demyelination;
- CI=
- confidence interval;
- CIDP=
- chronic inflammatory demyelinating polyneuropathy;
- CNTN1=
- contactin 1;
- GBS=
- Guillain-Barré syndrome;
- IgG4=
- immunoglobulin G4;
- IVIg=
- IV immunoglobulin;
- MS=
- multiple sclerosis;
- NF155=
- neurofascin-155;
- OR=
- odds ratio;
- PNS=
- peripheral nervous system
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* These authors contributed equally to this work.
Supplemental data at Neurology.org
Editorial, page 796
- Received May 7, 2015.
- Accepted in final form October 1, 2015.
- © 2016 American Academy of Neurology
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